Table 1.

Typical laboratory test results for VWD subtypes

NormalType 1Type 1CType 2AType 2B*Type 2MType 2NType 3
VWF:Ag ↓↓ ↓↓ N, ↓, or ↓↓ N, ↓, or ↓↓ ↓ or ↓↓ N or ↓ Absent 
VWF:RCo/VWF:Ag, VWF:GPIbM/VWF:Ag, VWFGPIbR/VWF:Ag ↓↓ ↓ or ↓↓ ↓↓ N/A 
FVIII/VWF:Ag ↓↓ N/A 
LD-RIPA None None None None ↑↑ None None None 
Platelet count N or ↓ 
VWF multimers ↓HMW ↓HMW Absent 
VWFpp/VWF:Ag ↑ or ↑↑ N or↑ N or ↑ N/A 
VWF:CB/VWF:Ag ↓↓ ↓ or ↓↓ N or ↓ N/A 
NormalType 1Type 1CType 2AType 2B*Type 2MType 2NType 3
VWF:Ag ↓↓ ↓↓ N, ↓, or ↓↓ N, ↓, or ↓↓ ↓ or ↓↓ N or ↓ Absent 
VWF:RCo/VWF:Ag, VWF:GPIbM/VWF:Ag, VWFGPIbR/VWF:Ag ↓↓ ↓ or ↓↓ ↓↓ N/A 
FVIII/VWF:Ag ↓↓ N/A 
LD-RIPA None None None None ↑↑ None None None 
Platelet count N or ↓ 
VWF multimers ↓HMW ↓HMW Absent 
VWFpp/VWF:Ag ↑ or ↑↑ N or↑ N or ↑ N/A 
VWF:CB/VWF:Ag ↓↓ ↓ or ↓↓ N or ↓ N/A 

HMW, high molecular weight; LD-RIPA, low-dose ristocetin-induced platelet aggregation; N, normal; ↓, reduced; ↓↓, markedly reduced; ↑, increased; ↑↑, markedly increased.

*

Platelet-type VWD has a similar laboratory phenotype.

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